Primary hyperaldosteronism resident survival guide: Difference between revisions

Revision as of 15:58, 17 July 2017

Template:Primary hyperladosteronism Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Overview

Causes

Common Causes

Common causes of Conn's Syndrome may be divided into:

Adrenal causes:
- Aldosterone-secreting adrenal adenoma (APA-benign tumor, 50-60%)
- Idiopathic hyperaldosteronism (IHA-Bilateral hyperplasia of the adrenal gland, 40-50%)
Extra-adrenal causes
- Ectopic secretion of aldosterone (Ovaries and Kidneys)

Less Common Causes

Familial hyperaldosteronism type I (glucocorticoid-remediable aldosteronism [GRA])
Familial hyperaldosteronism II (the familial occurrence of APA or bilateral idiopathic hyperplasia or both)
Familial hyperaldosteronism type III (associated with the germline mutation in the KCNJ5 potassium channel)
Pure aldosterone-producing adrenocortical carcinomas
Unilateral adrenal hyperplasia

Diagnosis and Treatment

Preferred screening population:

• Blood pressure > 160 / 100 particularly (< 50 years)
• Resistant hypertension or refractory hypertension (use of > 3 anti-hypertensives and poor control of blood pressure)
• Hypokalemia (provoked by diuretic therapy or unprovoked)
• Hypertension and incidentally discovered adrenal adenoma
• Hypertension with a family history of early-onset hypertension (< 20 years) or cerebrovascular accident at age less than 40 years

• Hypertensive first-degree relatives of patients with PA

Plasma Renin Activity/Aldosterone Ratio

Normal or High Renin (Plasma Renin/Aldosterone ratio <10

Suppressed Renin (Plasma Renin/Aldosterone ratio >20

*Renin-secreting tumors
*Diuretic use
*Renovascular hypertension
*Coarctation of aorta
*Malignant phase hypertension

Urinary aldosterone

Elevated

Normal

Low

Conn's syndrome (Primary aldosteronism)

Profound K+ depletion

• 17 alpha hydroxylase deficiency
• 11 beta hydroxylase deficiency
• Liddle's syndrome
• Licorice ingestion
• Deoxycortisone producing tumor

Add Mineralocrticoid antagonist for 8 weeks

BP response

No BP response

• Deoxycorticosterone excess( Tumor, 17 alpha hydroxylase and 11 beta hydroxylase deficiency)
• Licorice ingestion
•Glucocorticoid resistance

Liddle's syndrome)

@@ Line 20: / Line 20: @@
 *Unilateral adrenal hyperplasia
 ==Diagnosis and Treatment==
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+{{familytree/start}}{{familytree | | | | | | | | | A01 | | | | | |A01=Preferred screening population:<br>
+• Blood pressure > 160 / 100 particularly (< 50 years)<br>
 • Resistant hypertension or refractory hypertension (use of > 3 anti-hypertensives and poor control of blood pressure)<br>
-• Hypokalemia (provoked by diuretic therapy or unprovoked)<br>• Hypertension and incidentally discovered adrenal adenoma<br>• Hypertension with a family history of early-onset hypertension (< 20 years) or cerebrovascular accident at age less than 40 years<br>• Hypertensive first-degree relatives of patients with PA}}
+• Hypokalemia (provoked by diuretic therapy or unprovoked)<br>
+• Hypertension and incidentally discovered adrenal adenoma<br>
+• Hypertension with a family history of early-onset hypertension (< 20 years) or cerebrovascular accident at age less than 40 years<br>
+• Hypertensive first-degree relatives of patients with PA}}
 {{familytree | | | | | | | | | |!| | | | | | | | }}
 {{familytree | | | | | | | | | B01 | | | | | |B01=Plasma Renin Activity/Aldosterone Ratio}}